Sickle Cell Disease

Question 1

Priapism is _________.

Answer 1

the state in which the erect penis does not return to its flaccid state

Question 2

List the four effects that result from decreased NO activity (from hemolysis) in the arteries.

Answer 2

• priapism
• pulmonary hypertension
• leg ulcerations
• stroke

Question 3

Polychromasia refers to _______.

Answer 3

large RBCs that have a bluish tint – indicative of reticulocytes

Question 4

The most common complaint of those with sickle cell disease _________.

Answer 4

vaso-occlusive pain, a complication resulting in extensive pain from blocked blood vessels

Question 5

Acute chest syndrome is _________.

Answer 5

occlusion of capillaries in the lungs resulting in edema

Question 6

Sickle cell disease can result in splenic dysfunction, a state in which patients will be highly susceptible to _____.

Answer 6

infection from encapsulated bacteria (steptococcus pneumonaie, hemophilus influenzae, meningococcus)

Question 7

Aplastic crisis, another potential consequence of sickle cell disease, involves infection by ______.

Answer 7

parvovirus B19

Question 8

Splenic infarction happens when __________.

Answer 8

the capillary filters of the spleen get clogged and blood cannot flow through

Question 9

Large-vessel strokes affect ____ of those with sickle cell, but evidence suggests ________ may be more common.

Answer 9

10%; silent ischemic attacks

Question 10

Sickle nephropathy is present in _____ of those with SCD.

Answer 10

10% - 15%

Question 11

Retinopathy, in which _______, is present in 11% - 45% of those with SCD.

Answer 11

vaso-occlusion in the eye produces retinal detachment and hemorrhage

Question 12

_____ and _____ are also complications of SCD.

Answer 12

Avascular necrosis (resulting in bone atrophy); leg ulcers

Question 13

Although hydroxyurea can help reduce acute pain and acute chest, it does not _______.

Answer 13

reduce chronic organ damage

Question 14

Abnormal lab values in sickle cell anemia include _______.

Answer 14

increased WBC, RDW, and retic; abnormal smear

Question 15

In SC patients, parvovirus B19 causes ______.

Answer 15

aplastic crisis due to impaired bone marrow response

Question 16

Why do SC patients often have bilirubin gallstones?

Answer 16

Because they have chronically elevated bilirubin

Question 17

People with SC are susceptible to __________.

Answer 17

stroke (10%), PAH (due to capillary occlusion in the lungs), and splenic infarction

Question 18

Causes of SC crisis include _______.

Answer 18

inflammation, deoxygenation,

Question 19

Less than ______ have an eligible bone marrow donor.

Answer 19

20%